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We report a case of malignant solitary fibrous tumor involving the pineal region in a 49-year-old woman. The patient presented with headache, slowly progressive weakness of the right lower extremities and upgaze palsy over the past year. Histologically, the tumor was composed of moderately hypercellular proliferated spindle cells with eosinophilic collagen bands. These cells were diffusely and strongly immunoreactive with CD34, CD99, and vimentin, but were negative with epithelial membrane antigen, S-100 protein, Bcl-2, smooth muscle actin, cytokeratin and glial fibrillary antigenic protein. MIB-1 labeling indices and mitosis rates were 7.3 +/- 1.8% and 5 per 10 high power fields, respectively. Ultrastructural examination revealed that the neoplastic cells had features of fibroblastic differentiation. Differential diagnoses included fibrous meningioma and hemangiopericytoma. The present case provides one unique example of a rare entity to the already diverse spectrum of the pineal region neoplasms encountered in neuropathology.

Pineal tumors are rare neoplasms that are categorized into pineoblastomas, pineocytomas, and pineal parenchymal tumors of intermediate differentiation. Pineal anlage tumors are primary pineal tumors with neuroepithelial and ectomesenchymal differentiation and without endodermal differentiation. The authors review the literature and report the case of a 5-month-old boy with a pineal anlage tumor. This is only the sixth case of a pineal anlage tumor reported in the English-language literature adding to the understanding of this tumor's presentation, immunomorphological and molecular characteristics, embryological origin, radiological appearance, treatment outcome, and prognosis.

Objective: To review the clinical manifestations, imaging, tumor markers, treatment methods, pathology results and clinical curative effects of pineal region tumors and to evaluate the characteristics and intervention strategies for those tumors., Methods: The clinicopathological data of 132 patients with pineal region tumor treated in our department between January 2000 and May 2008 were retrospectively studied., Results: A moderate predominance in males was presented. The clinical manifestations of the disease included increased intracranial pressure and ocular movement impairment. There were some features but no regularity and specific appearance on imaging including CT and MRI. 88.6% of patients associated with hydrocephalus. A high serum level of alpha-fetoprotein (AFP) was presented in 14 cases and high HCG in 9 cases. Eighteen cases received direct radiation therapy and 7 had radiotherapy post biopsy. 107 cases were treated surgically and 63 cases received postoperative adjuvant treatment. 114 cases had pathology results including 56 germ cell tumors. The patients were followed up for 12 approximately 132 months. Recurrence developed in 23 cases and 12 cases died. The 5-year survival rate was 89.3%., Conclusion: Pineal region tumors are often associated with hydrocephalus and this makes preoperative diagnosis difficult. Imaging examination may help diagnosis but less specific. Germ cell tumors may diagnosed by some tumor markers. Radiation therapy is the choice of treatment for pure germinomas. Other types of pineal region tumors should receive surgical treatment. Postoperative adjuvant treatment based on pathology can provide a good prognosis in pineal region tumor.

Purpose: To evaluate the efficacy of interstitial radiosurgery (IRS) for pineal parenchymal tumors (PPTs)., Patients and Methods: 18 consecutively admitted patients (twelve male and six female, age range 6-68 years, median age 34 years) with PPTs (eight pineocytomas, ten malignant PPTs) were treated at the authors' institution with IRS using stereotactically guided iodine-125 seed implantation ((125)I-IRS) as either primary or salvage therapy. The cumulative tumor surface dose ranged from 40 to 64 Gy. Adjuvant radiotherapy of the whole brain or the craniospine was done in patients with grade III and grade IV PPT. The median follow-up period was 57.4 months (range 6-134 months)., Results: Overall actuarial 5- and 8-year survival rates after IRS were 100% and 86% for pineocytomas, and the overall actuarial 5-year survival rate was 78% for high-grade PPTs. Follow-up magnetic resonance imaging showed complete remission in 72% (13/18) and partial remission in 28% (5/18) of the cases. One patient developed an out-of-field relapse 4 years after partial remission of a pineocytoma, which had already been treated with IRS. There was no treatment-related mortality. Treatment-related morbidity occurred in two patients only., Conclusion: This study indicates that stereotactic (125)I-IRS for the management of PPTs is quite efficient and safe. Due to the low rate of side effects, IRS may develop into an attractive alternative to microsurgery in de novo diagnosed pineocytomas. In malignant PPTs, IRS may be routinely applied in a multimodality treatment schedule supplementary to conventional irradiation.

Objective: To identify clinical and pupillographic features of patients with a relative afferent pupillary defect (RAPD) without visual acuity or visual field loss caused by a lesion in the dorsal midbrain., Design: Experimental study., Participants and Controls: Four patients with a dorsal midbrain lesion who had normal visual fields and a clinically detectable RAPD., Methods: The pupil response from full-field and hemifield light stimulation over a range of light intensities was measured by computerized binocular pupillography., Main Outcome Measures: The mean of the direct and consensual pupil response to full-field and hemifield light stimulation was plotted as a function of stimulus light intensity., Results: All 4 subjects showed decreased pupillographic responses at all intensities to full-field light stimulation in the eye with the clinical RAPD. The pupillographic responses to hemifield stimulation showed a homonymous pattern of deficit on the side ipsilateral to the RAPD, similar to that observed in a previously reported patient with an optic tract lesion., Conclusions: The basis of a midbrain RAPD is the nasal-temporal asymmetry of pupillomotor input that becomes manifest when a unilateral postchiasmal lesion interrupts homonymously paired fibers traveling in the contralateral optic tract or midbrain pathway to the pupillomotor center, respectively. The pupillographic characteristics of an RAPD resulting from a dorsal midbrain lesion thus resemble those of an RAPD resulting from a unilateral optic tract lesion, but without the homonymous visual field defect., Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article., (Copyright 2010 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Metastatic Neuroendocrine Tumor (NET) to the pineal gland is a unique manifestation previously unreported in the literature. We describe an unusual case of metastatic bronchial NET to the pineal gland in a 71-year-old male patient. His primary NET had been resected six years previously and there was no indication of the presence of disseminated metastatic disease at that time. Due to increased uptake by the pituitary gland on the post-operative 111Indium-pentetreotide scintigraphy (Octreoscan), an intra-sellar mass was diagnosed and excised using a transsphenoidal approach; histology revealed an unrelated non-functional pituitary macroadenoma. Four years later, a new mass appeared on MRI, involving the pineal gland, and was diagnosed on biopsy as a metastatic lesion from the original bronchial NET. Since this lesion was not accessible to surgery, it was treated successfully with radiosurgery. The case suggests that NETs should be considered in the differential diagnosis of pineal gland metastases and that radiosurgery may be an effective alternative in the treatment of these patients.

Papillary glioneuronal tumor (PGNT) is a newly described mixed glioneuronal tumor, recently included in the World Health Organization classification of central nervous system tumors. We report morphologic and immunohistochemical characteristics PGNT of the pineal region extending into the third and lateral ventricles in a four-year-old male child and reviewed all the cases reported in the international literature till date. In this child the diagnosis of PGNT was established by endoscopic biopsy and squash smear examination.

Objective: Primary melanomas of the pineal region are exceedingly rare and may be difficult to diagnose. Clinical, radiological and pathological features as well as diagnostic procedures are discussed. CASE HISTORY We report herein on a 44-year-old man who presented with uncontrolled epileptic seizures. Magnetic resonance imaging revealed a pineal mass hyperintense on T1-weighted and isointense on T2-weighted sequences with diffuse leptomeningeal involvement and intense homogeneous contrast enhancement after gadolinium administration. A frontal leptomeningeal and cortical biopsy was performed. Histological examination showed a malignant melanocytic tumor cell proliferation expressing Melan-A, but not HMB-45 or S100 protein. Even if we have no proof that the tumor actually arose in the pineal gland, based on the radiological and histological findings, and on the unremarkable dermatologic and ophthalmologic examinations, a primary pineal melanoma with leptomeningeal dissemination was diagnosed. The patient received temozolomide-based chemotherapy followed by whole brain irradiation. The patient died 52 weeks after disease onset and 13 weeks after treatment initiation., Conclusion: A diagnosis of pineal melanoma should be considered in the presence of a pineal mass that appears hyperintense on T1-weighted images and hypo- to isointense on T2-weighted images. The diagnosis is provided by pathological examination of tumor specimens obtained at surgical resection or at leptomeningeal biopsy. However, immunochemistry using anti-Melan-A, -S100 protein and/or -HMB45 antibodies on cerebrospinal fluid and leptomeningeal samples may be helpful in diagnosing such a disease. The prognosis of primary pineal melanoma is variable but meningeal spreading carries a dismal prognosis. The best therapeutic management is yet to be defined.

Pineal gland tumors are rare and account for less than 1% of all primary brain tumor diagnoses. They are more commonly seen in pediatric patients than in adults. We analyzed the available SEER data on pineal gland tumors that were diagnosed during the period 1973-2005. The cohort was subdivided into groups on the basis of tumor histology: germ cell tumors, pineal parenchymal tumors, gliomas, and other pineal tumors. Analyses of incidence, survival, factors influencing survival, and treatment modalities are provided. Among the 633 patients with pineal tumors, male sex was predominant, i.e., sex ratio was 3:1 for the whole group and 11.8:1 for those with germ cell tumors. The 5-year overall survival (OS) for the cohort was 65% +/- 2.1%. Those with germ cell tumors experienced the best survival (OS = 78.9% +/- 2.3%), followed by those with gliomas (OS = 61% +/- 9.3%), and those with pineal parenchymal tumors (OS = 47.2% +/- 4.2%). Non-germ cell tumors, absence of radiotherapy from treatment regimen, and diagnosis before 1993 were the only factors associated with a negative impact on survival. The extent of surgical tumor resection did not affect survival in any histologic subgroup. We conclude that, although pineal tumors are histologically diverse, they share some similarities due to their unique location. An aggressive surgical approach should be considered with caution in this region. Further studies on different pineal tumors subtypes are needed.

Papillary tumor of the pineal region (PTPR) is an uncommon tumor recently added to the WHO classification of CNS tumors. We report a case of PTPR in a young boy that was noteworthy for early CSF dissemination and relentless progression. In spite of intensive chemotherapy and comprehensive radiotherapy, the boy died. The neuroimaging appearance is unique with possible multicentric origin of the tumor and intense uptake of (111)In-DTPA-pentetreotide.

The deep-seated location of pineal parenchymal tumors (PPTs) and their associations with critical structures make their surgical resection technically challenging; further, the rarity of PPTs and repeated changes in their histopathological diagnostic criteria makes the study of their biological behavior and clinical outcomes difficult. Here, we describe the surgical techniques and results of an occipital transtentorial approach for PPTs together with the results in the clinicopathological study of PPTs. Since 1982, we have treated 93 patients with pineal region tumors, including 17 PPTs, with the occipital transtentorial approach using the lateral semiprone position. The infrasplenial approach is helpful in separating the internal cerebral veins from the tumor, particularly when the tumor is tightly adherent to the veins. Permanent homonymous hemianopsia occurred in 1 of the 17 patients with PPTs. Permanent ocular movement disorders were not encountered. Extensive removal of the tumor significantly prolongs survival at least in patients with pineocytomas and PPT of intermediate differentiation (PPTIMD). Despite extensive resection and adjuvant radiochemotherapy, the prognosis of the patients with pineoblastomas is extremely poor. Although the proliferative potentials of pineocytomas and PPTIMD were significantly lower than those of pineoblastomas, there was no such difference between pineocytomas and PPTIMD., (Copyright (c) 2009 S. Karger AG, Basel.)

With the development of diagnostic, radiologic, and therapeutic modalities, strategies for management of central nervous system (CNS) germinoma are changing gradually. The author advocates that typical germinomas can be diagnosed based on their typical clinical and radiological findings, together with slight elevation of beta-human chorionic gonadotropin levels in the serum and/or cerebrospinal fluid (CSF) and quick response to radiation or chemotherapy. Radiation therapy has been the standard treatment for CNS germinoma until recently. Germinomas 4 cm or less in diameter can be cured with radiation doses of 40-45 Gy. Regarding the treatment volume, an individualized approach is recommended and a focal radiation field covering at least major parts of the ventricular system is recommended if no CSF dissemination is present and CSF cytology is negative. Such irradiation is best given by intensity-modulated radiation therapy. Systemic chemotherapy with reduced doses (24-30 Gy) of radiation has to some extent been successful, but longer follow-up periods are necessary to draw conclusions regarding the superiority of this treatment over standard-dose radiation therapy. CNS germinoma patients should be completely cured with minimum morbidity, probably by employing appropriate doses of chemotherapy and intensity-modulated radiation therapy in the future., (Copyright (c) 2009 S. Karger AG, Basel.)

Positron emission tomography (PET) with (18)F-fluorodeoxy-glucose indicates metabolically active tissue. When investigating enhancing intracranial tumours, we have suggested that PET positivity might suggest an intracranial germ cell tumour (IGCT). Here, we present a case with dicentric IGCT where PET was initially discordant between the lesions and where PET then became negative despite clearly aggressive clinical behaviour. A cautionary note is introduced with respect to the interpretation of negative (18)F-FDG PET when investigating enhancing intracranial lesions., (Copyright 2009 S. Karger AG, Basel.)

We report a boy who in 1994, at the age of 11, presented with headache and vomiting. The fi nal diagnosis was papillary tumor of the pineal region (PTPR). Magnetic resonance imaging (MRI) revealed a heterogeneous mass. Hydrocephalus was addressed by immediate ventricle drainage; subsequently, we attempted tumor removal. As the intraoperative diagnosis of the hemorrhagic tumor was primitive neuroectodermal tumor (PNET), we did not proceed to total removal. After the delivery of radiotherapy (50.4 Gy) and one course of Nimustine hydrochloride (ACNU) chemotherapy, the residual tumor was completely resected. The diagnosis at that time (1994) was papillary pineocytoma. He was followed on an outpatient basis for 15 years and remained free of recurrence. This type of tumor was later proposed to represent a new distinct tumor subtype, papillary tumor of the pineal region (PTPR). Our data indicate that our patient's tumor should be included in this category.

The treatment for the pineal region tumors depends on tumor histology. Nowadays, germinomas can be cured by radiotherapy and chemotherapy without surgical resection but the other pineal region tumors should be primary treated by surgery. Two microsurgical approaches, the infratentorial supracerebellar and the occipital transtentorial, are accepted as the main standard accesses to the pineal region. For benign pineal tumors (pineocytoma, meningioma, mature teratomas, symptomatic pineal cysts, etc.) radical surgical resection can be curative. For malignant tumors radical surgical resection is not an objective. Serum and CSF markers contribute to the diagnosis of pineal parenchymal tumors. b-HCG is mainly positive in choriocarcinomas, embryonal carcinomas and mixed germ cell tumors and AFP is expressed by yolk sac tumors, embryonic carcinomas, immature teratomas and mixed germ cell tumors, b-HCG is usually low in germinomas which are often positive for PLAP on immunohistochemistry. Fifty-one pineal region tumors were surgically treated by senior author (NdT). Only 17 of them were the neoplasms originating from pineal body (pineal tumors). In conclusion it can be stressed that management of pineal tumors requires a multidisciplinary cooperation. With the exception of germinoma where only a biopsy is needed, the role of the surgeons still remains prominent as resection of pineal tumors requires high technical skill and experience as well as precise clinical judgment.

A 20-year-old man presented with a rare case of germinoma with a large component of epithelioid cell granuloma manifesting as oscillopsia. Magnetic resonance imaging demonstrated a mass in the pineal region with homogeneous enhancement with gadolinium. Craniotomy was performed, ending in biopsy. The initial histological diagnosis was epithelioid cell granuloma, but systemic investigation detected no evidence of granulomatous disorder. A revised diagnosis of germinoma was based on positive immunohistochemical staining for placental alkaline phosphatase (PLAP) and c-kit. Histological diagnosis is sometimes incorrect if granulomatous reaction is dominant. Immunohistochemical staining for PLAP and c-kit should be performed if germinoma is clinically suspected.

Primary germ cell tumors (PGCT) of the central nervous system usually develop in the third ventricle area, and most frequently in the pineal region. The suprasellar region is the second preferential site for development of these tumors which are rarely simultaneously present in these two sites. We report five new cases of PGCT with pineal and suprasellar localizations, which appeared in late puberty in four boys and one girl aged 17-19 years. The clinical picture associated signs of intracranial hypertension, convergence and verticality palsies, diabetes insipidus and pituitary deficiency. Encephalic MRI revealed a double localization. Endocrine tests revealed a particular pattern associating central diabetes insipidus and a hypothalamic-pituitary disconnection syndrome. Following identification of the pathological type of lesions via a neurosurgical approach, treatment was based on a combined method using chemotherapy, radiotherapy and hormone replacement. Based on this treatment, prolonged remissions were obtained with a good quality of life.

Objects: The goal of the study was to establish if endoscopic biopsy during third ventriculostomy contributes to morbidity in the management of paediatric pineal region tumours presenting with hydrocephalus., Materials and Methods: This study was a retrospective descriptive study in patients less than 18 years of age who have presented with a pineal region tumour between 2000 and 2006. Data were obtained from case notes. Twelve patients had presented with a pineal region mass with symptomatic hydrocephalus. Of these, eight had endoscopic biopsy during third ventriculostomy., Conclusions: No mortality or permanent morbidity was seen following endoscopic biopsy. Two cases of transient worsening of pre-operative diplopia were noted. Diagnostic sensitivity for endoscopic biopsy is 75%. Tumour markers were not significantly raised in any plasma and cerebrospinal fluid samples. Endoscopic biopsy during third ventriculostomy in paediatric pineal region tumours is safe and results in good diagnostic yields. It should play an integral role in the initial management of patients in this setting.

Pineal parenchyma tumor of intermediate differentiation (PPTID) is a very rare intracranial tumor, and pathological investigation limited to immunohistological and ultrastructural analyses have been published to date. Although intraoperative cytology is one of the important approaches for initial diagnosis in brain tumors, no or little studies on cellular morphology of PPTID have been demonstrated due to its rarity. We report here cytological features of PPTID obtained from stereotactic surgical specimens in a case of 27-year-old female manifested by dizziness and diplopia. Brain MRI revealed an unhomogeneously enhanced, large-sized tumor (56 x 52 x 60 mm) mainly located in the pineal region expanding from the midbrain to superior portion of the cerebellum and the fourth ventricle. Squash cytology showed increased nucleocytoplasmic ratio, hyperchromatic nuclei, and small rosette-like cell cluster but cellular pleomorphism was mild to moderate and necrotic background was not observed. Histology showed high cellularity, moderate nuclear atypia, and small rosette formation but neither bizarre tumor cells nor necrosis was present. Mitotic counts were very low (less than 1 per 10 high-power fields) and the MIB-1 labeling index was relatively high (10.1%). Tumor cells were immunohistochemically positive for neural markers such as synaptophysin, neurospecific enolase but not for glial fibrillary acidic protein or S-100. In some parts, cells were strongly reactive for neurofilament protein. Taken together, we made a final diagnosis of PPTID. This is the first presentation of cytological analysis by squash preparation that gives an important clue to accurate diagnosis of pineal parenchymal tumor and to understand its malignant potential.

Pinealgland tumors comprise 0.5%-1% of adult brain tumors. Pineoblastomas constitute less than half of these pineal gland tumors. Due to the key anatomic location, these tumors produce hydrocephalus by pressing on the aqueduct of Sylvius. Headache is a very common symptom. We describe the case of a 20-year-old female who presented with new onset persistent headache. Workup revealed a pineoblastoma. These tumors can be detected by MRI and diagnosed on cytology of cerebrospinal fluid (CSF) showing blue round tumor cells with specific tumormarkers. Combined chemo radiation therapy is the treatment for these tumors. New onset persistent headaches are present in 50%-60% of intracranial tumors. All new onset persistent headaches should be promptly evaluated.

The significance of histological diagnosis was retrospectively investigated in the treatment of 79 consecutive patients with pineal region tumours. Histological diagnoses were consistent with clinical diagnoses in 38 out of 39 patients with germ cell tumours and in all eight patients with non-germ cell tumours. The single exception was pineoblastoma. Forty-one patients were treated under a clinical diagnosis of pineal germ cell tumour using neoadjuvant chemotherapy and radiation therapy without histological verification. Of the 41 patients, 32 achieved an excellent response, whereas nine required the delayed resection of a residual tumour. Of these nine tumours, seven were mature or immature teratomas or scars. These patients had elevated levels of tumour markers. Most cases of pineal germ cell tumour could be identified on the basis of clinical findings only, and could be treated appropriately using neoadjuvant chemotherapy and radiation therapy without histological diagnosis. However, second-look surgery should be considered in cases with residual teratomatous components or unusual responses.

Tumors of the pineal region are uncommon, comprising approximately 0.4-1% of all intracranial tumors in adults in European and American series. Histopathologically, they are a very heterogeneous group of tumors. Of genuine pineal tumors, pineal parenchymal tumors of intermediate differentiation (PPTIDs) are the least frequently found type. In this paper, we report on the case of a patient with an unexpected and difficult-to-diagnose PPTID. A 2.2 x 2.2-cm midline mass within the posterior part of the third ventricle with consecutive obstructive hydrocephalus was found in a 44-year-old man presenting with diplopia and gait disturbances. There was no clear connection of the tumor to the pineal gland. Differential diagnosis included all intraventricular and midline tumors, therefore a biopsy was taken. Preliminary histopathological diagnosis was germinoma or primitive neuroectodermal tumor, and the tissue sample was reexamined by a referential neuropathological institute. Final diagnosis was PPTID. The tumor was then resected through a transventricular/transchoroidal approach. Histopathological examination of tumor specimen confirmed the diagnosis of a PPTID. Postoperatively, the patient received gamma-knife radiosurgery. At 1-year follow-up, there are no signs of tumor regrowth. Diagnosis of pineal parenchymal tumors in general and PPTIDs in particular can be troublesome. Their histopathological features are still being defined, as is the biological behavior of the different tumor entities. Thus, treatment options including surgery, radiation therapy, and chemotherapy remain controversial. We recommend surgical removal of PPTID, preferably in toto whenever the size of the tumor permits that kind of excision.

Several new entities of central nervous system tumors have been established in the past decade. By reviewing our cases and previous reports, we describe 4 neuronal and mixed neuronal-glial tumors (papillary glioneuronal tumor, extraventricular neurocytoma, rosette-forming glioneuronal tumor of the fourth ventricle, and glioneuronal tumor with neuropil-like islands), angiocentric glioma and papillary tumor of the pineal region, with an attempt to explain the pathological basis of the imaging features.

Pineoblastomas are uncommon pineal tumors, which demonstrate rapid growing and poor prognosis. We report the case of a 43-year-old man with an enhancing pineal region mass, which showed restriction of the diffusion on diffusion-weighted (DW) MR images. The surgical biopsy defined the diagnosis of pineoblastoma and the therapy was initiated with radiation and chemotherapy. Three months later, the follow-up MR imaging showed areas suggestive of necrosis and the DW images demonstrate no significant areas of restricted diffusion. The differential diagnosis of pineal region masses that could show restriction of diffusion is discussed.

A 61-year-old woman had suffered from severe headache and nausea over 20 times during the last 43 years. An subarachnoid hemorrhage (SAH) was detected by spinal puncture in some other hospitals, but the source of hemorrhage remained unknown in spite of repeated angiography. At the age of 61, she was diagnosed as having normal pressure hydrocephalus, and received a ventriculo-peritoneal shunt. She suffered from sudden headache 12 days after surgery. A CT scan showed a SAH and enlargement of the pineal mass. The tumor was totally removed via the occipital interhemispheric transtentorial approach and was diagnosed histologically as a pineocytoma. She has been free from SAH for three years since removal of the tumor. Pineal apoplexy should be considered as a cause of SAH.

Introduction: Acoustic neuroma usually presents as an unilateral tumor, seldom - bilateral and rarely in coexistence with other central nervous system neoplasms. The following paper reports such a case of a 21-year-old male patient presented with sudden deafness in left ear accompanied with tinnitus and vertigo. Symptoms started 4 weeks prior hospitalization. Their aggravation has been observed 7 days before admission to the hospital. Audiometry revealed moderate sensorineural hearing loss in left ear (for low and middle frequencies), brainstem auditory evoked potentials were absent on the left side and ENG examination showed left peripheral vestibular impairment. Initially patient received i.v. vasodilatators showing 20-25 dB improvement in low frequencies after 3 days of treatment. MRI study revealed in the left internal acoustic meatus mass (7 x 7 x 14 mm) suggesting acoustic neuroma and an oval mass (7 x 9 x 14 mm) in the pineal gland presenting radiological features of pinealoma. Patient has been qualified for neurosurgical treatment. Acoustic neuroma has been removed by suboccipital approach and pinealoma has been left for further observation as it was found incidentally. Histopathological examination confirmed diagnosis of left VIII nerve schwannoma. The left facial palsy (House-Brackmann III/IV grade) and profound hearing loss appeared after surgery. The postoperative course shows no evidence of acoustic neuroma recurrence.

Background and Purpose: Pineocytomas have been described as well-circumscribed, homogeneously enhancing masses. However, there is considerable variability in this appearance, and certain pineocytomas may have a predominantly cystic appearance on imaging. This has led some to suggest that differentiation between pineocytomas and pineal cysts may not be possible. We have attempted to determine if cystic pineocytomas could be found in a series of these tumors evaluated by CT and MR imaging., Materials and Methods: We searched the radiology literature as well as the medical records from our own institution for pathologically proved pineocytomas with available preoperative imaging or imaging reports, with specific focus on whether postcontrast MR imaging was included. In cases in which images were available, they were evaluated by a Certificate of Added Qualification (CAQ)-certified neuroradiologist and a radiology resident, who attempted to determine if the pineocytomas had any MR imaging characteristics of typical pineal cysts. To be considered a typical pineal cyst, an area of signal-intensity abnormality must be centered on the pineal recess, demonstrating internal homogeneity on T2-weighted imaging, following CSF signal intensity on T1- and T2-weighted images, without any marginal lobularity or nodular contrast enhancement and a wall thickness of <2 mm. In cases in which imaging was not available, radiology reports and/or descriptions provided in the radiology literature were reviewed by a CAQ-certified neuroradiologist. For any lesion described as cystic, we again attempted to elucidate the exact extent of imaging that was performed, note specific lesion characteristics, and determine if the lesion met the criteria described previously. Finally, for tumors in which image size was provided, the mean value of maximal tumor dimension, SD, median, and range were calculated., Results: Forty-four pathologically proved cases of pineocytomas from the radiology literature, as well as 8 pathologically proved cases of pineocytomas from our institution with available imaging studies and/or reports, were reviewed. Of these, 23 were solid masses, and 7 were partially solid and cystic, whereas 14 tumors could not be completely characterized due to incomplete imaging evaluation. Eight were primarily cystic; however, none of these could be confidently characterized as meeting the criteria for a typical cyst., Conclusion: In our analysis, no truly cystic pineocytomas were identified.

A 48-year-old female presented with an extremely rare primary tumor of the pineal region with papillary features manifesting as morning headaches persisting for 1 month. Magnetic resonance imaging showed a well-defined mass, with some cystic components, in the region of the pineal gland. The tumor was completely removed through an occipital transtentorial approach in the prone position. Histological examination found a distinctive papillary growth pattern in which the vessels were covered by multiple layers of tumor cells. The histological diagnosis was papillary tumor of the pineal region (PTPR), which has recently been described as a distinct clinicopathological entity requiring careful follow up because the prognosis is not well understood. Postoperatively, the patient has continued to do well, with no recurrence at the 8-month follow-up examination. PTPR should be considered in the differential diagnosis of pineal tumors. PTPR may have been frequently misinterpreted in the past as either ependymoma or choroid plexus papilloma due to the similar morphology.

A case of 19-year-old male with idiopathic diabetes insipidus diagnosed 9 years ago. 1.5 years from the onset of the disease vision disturbances, neurologic deficiencies and symptoms of hypopituitarism showed up. MRI examination revealed an advanced hypophyseal and pineal gland tumor--germinoma. Total regression was achieved with radio- and chemotherapy. For 7 years from the end of treatment patient has not declared any complains except for vision disturbances and hypopituitarism has been substituted successfully. The case puts on the necessity of a strict endocrinologic and radiologic follow-up in patients with idiopathic diabetes insipidus due to the possibility of existing potentially curable disease ie. intracranial tumor.

Objective: We report two rare cases of primary choriocarcinoma in the pineal region verified histologically. In both cases, the pre-operative serum level of human chorionic gonadotropin (HCG) was significantly elevated to 128-/+935.7 and 9 -/+088.9 mIU/ml, respectively, and serum alpha-fetoprotein (AFP) was negative. The tumors were microsurgically removed, and postoperative hydrocephalus were treated by endoscopic third ventriculostomy. Both patients underwent chemotherapy and radiotherapy. After adjunctive treatment, the serum HCG decreased within normal range. During the two-year-long follow-up, no radiological (MRI) evidence was found to suggest recurrence in MR imaging, and the serum HCG was normal in one patient, but mildly elevated in the other. HCG measurement can be crucial to the diagnosis and post-treatment monitoring of choriocarcinoma, and radical surgical tumor removal and combined modality therapy including chemotherapy and radiotherapy may ensure good results.

The authors report a case of a recurrent intracranial germinoma along the site of an endoscopic third ventriculostomy (ETV) after complete local tumor control using 3D conformal radiation therapy. A 13-year-old girl presented with sudden left upward gaze limitation for 4 days. A pineal region tumor and obstructive hydrocephalus were noted on magnetic resonance (MR) images. An ETV and tumor biopsy procedure were performed, which revealed the lesion to be a germinoma. The patient's visual symptoms and hydrocephalus disappeared postoperatively. Chemotherapy using cisplatin, etoposide, vincristine, and cyclophosphamide was initiated on postoperative Day 10. An MR image obtained 10 weeks after surgery and 2 weeks after chemotherapy revealed a significant (> 50%) reduction of the lesion. Radiation therapy was administered at 50.4 Gy to the target and 36 Gy to the periphery. Ten months after surgery, an MR image revealed further shrinkage of the tumor mass. One year after surgery, follow-up MR imaging demonstrated a small mass lesion at the entry site of the ETV, measuring 1.0 x 1.4 x 1.5 cm. An operation was performed to remove the small lesion, and pathological findings revealed it to be of the same histology as the primary tumor.

Background: Papillary pineocytoma is an extremely rare tumor usually with a poor outcome., Case Report: We report a case of a 10-year-old-girl with pineal gland tumor and obstructive hydrocephalus diagnosed using MRI. The child was successful treated by insertion of a ventriculoperitoneal shunt and consecutive tumor resection by supracerebellar-infratentorial approach. Histopathological examination showed a papillary structure of the pineocytoma. As such, tumors are considered to be aggressive the child was subjected to radio- and chemotherapy., Conclusion: At six year follow-up after surgery, the patient is symptom-free and the MRI shows no tumor recurrence.

Objective: To describe the relationship between symptomatology and time to diagnosis of an institutional series of patients with CNS germ cell tumor (CNSGCT) over a 16-year period., Methods: Thirty consecutive patients newly diagnosed with CNSGCT (mean age 10.9 years; range 6 to 17 years; 70% boys) were evaluated at our institution between 1990 and 2006., Results: Duration of symptoms prior to diagnosis ranged from 5 days to 3 years (mean 8.4 months). Tumor location included pineal (14), suprasellar (8), pineal/suprasellar (3), pineal/thalamic (4), and basal ganglionic/thalamic (3). Five patients had disseminated disease at the time of diagnosis. Features including headache, nausea, vomiting, and visual changes led to earlier diagnosis. Symptoms including movement disorders, enuresis, anorexia, and psychiatric complaints delayed diagnosis in 9 of 30 patients, diagnosed 7 months to 3 years (mean 22.3 months) from symptom onset. In 7 of 9 patients with delayed diagnosis, enuresis was present. Seventeen of 30 patients had signs of endocrine dysfunction at presentation that included diabetes insipidus (4), hypothyroidism (8), and growth hormone deficiency (4). Ophthalmologic findings of decreased visual acuity, visual field deficits, or ocular abnormalities were present in 13 patients. Duration of symptoms did not correlate with tumor subtype or event-free survival. In three patients with basal ganglionic/temporal lobe, thalamic, or pineal/suprasellar signal abnormalities on MRI, neuroradiographic diagnosis was difficult., Conclusions: Diagnosis of CNS germ cell tumor is often delayed, and presentation may include movement disorders or mimic psychiatric disease. MRI interpretation can be challenging and may require serum/CSF markers and biopsy for diagnosis.

Objective: The pineal region is a rare intracranial site for metastasis. We report three patients initially considered to have metastatic papillary adenocarcinoma to the pineal region. On review, these papillary, keratin-positive neoplasms meet the criteria for papillary tumor of the pineal region (PTPR)., Clinical Presentation: These neoplasms occurred in three women (age range, 37-55 yr). Imaging studies demonstrated well-circumscribed lesions in the pineal region. All patients presented with obstructive hydrocephalus and symptoms attributable to hydrocephalus and tectal compression., Intervention: All three patients underwent near total microsurgical resection of the pineal region neoplasm, followed by adjuvant radiotherapy. The two patients with long-term follow-up (56-60 mo) have remained clinically stable without evidence of local or distant recurrence. The first two patients were initially diagnosed as having papillary metastatic carcinoma of unknown origin. The third patient was treated after the recent description of PTPR and met the histopathological diagnostic criteria. Retrospective pathological review of the previous two patients resulted in designation as PTPR., Conclusion: The morphological features of the tumors in our series, along with the clinical presentations, are similar to those in the original description of the PTPR. Our findings agree with the original hypothesis that the cells composing the PTPR are similar to ependymal cells of the subcommissural organ, thus furthering the hypothesis that the PTPR derives from a specialized ependymocyte associated with the subcommissural organ. The two patients with long-term follow-up (56-60 mo) have remained clinically stable without evidence of local or distant recurrence.

Exploration in neuroendoscopic surgery is occasionally insufficient because of the structural limitations in endoscope. It is not easy to identify the precise position of the endoscope during an operation. We here show a coaxial technique for neuroendoscopic exploration with monitoring by means of a microfiberscope. The endoscopic coaxial technique consists of two scopes. The microfiberscope has a diameter of 0.75 mm, flexible body and high-quality image system. We tested its ability to visualize the subject at several distances and applied this coaxial technique in neuroendoscopic surgery. The microfiberscope not only visualizes the object but also magnifies it by a distance. The scope was easy to handle with monitoring of its movement in the endoscopic view. The technique allowed us to safely explore details which are difficult to approach with an ordinary fiberscope or rigid-rod scope. The microfiberscope also adjusts to the neuronal anatomy and many clinical situations. Taken together, the microfiber coaxial technique might offer new advantages to modern neuroendoscopic surgery.

Tumors of the pineal region represent a diverse collection of tumors with a variety of natural histories. This diversity necessitates accurate histologic diagnosis to allow rational therapeutic planning. Evaluation of a pineal lesion should begin with craniospinal MRI and analysis of the cerebrospinal fluid (CSF). Whereas certainty of the histologic diagnosis is now a requirement for treatment in Western nations, some Asian centers continue to recommend a test dose of radiation therapy based on the high incidence of germinoma in those countries. If there is high clinical suspicion of a germinoma or tectal glioma, stereotactic or endoscopic biopsy may be pursued. All other lesions should be referred for open biopsy with microsurgical techniques. This approach provides adequate tissue for diagnosis, may be curative in low-grade tumors, and may substantially improve survival in patients with malignant tumors. If open surgery is not desired by the patient or practitioner, stereotactic or endoscopic biopsy may be followed by radiosurgery for localized, well-demarcated tumors. Radiation therapy is the first-line therapy for germinomas. Although the optimal radiation dosage and volume have not been decided, the current Children's Oncology Group trial may offer definitive evidence to address this dilemma in germ cell tumors. Evidence of CSF seeding requires craniospinal radiation and adjuvant chemotherapy regardless of tumor type. Diagnosis of any of the malignant tumors (non-germ cell tumors, pineoblastomas, and parenchymal tumors of intermediate determination) also requires craniospinal radiation (with local tumor doses of at least 50 Gy) and adjuvant chemotherapy (generally platinum based). Patients with tectal gliomas may undergo excision with or without postoperative radiation; however, they also may be observed with vigilant follow-up alone.

Aims: To compare the suitability of new seminoma markers including transcription factors AP-2gamma, OCT3/4 and M2A for detection of metastatic and extragonadal seminomas with the two well-known markers c-KIT and PLAP., Materials and Methods: The immunohistochemical distribution of PLAP, c-KIT, M2A, AP-2gamma and OCT3/4 was examined in two pineal germinomas, 28 metastatic seminomas and 10 of their testicular primaries. Evaluation of specificity was achieved by additional tissue array studies on 75 malignancies other than germ cell tumours (GCT). Clinical data including serum PLAP were available in 18 patients., Results: Compared with other markers, significantly better staining results were observed with antibodies to M2A and AP-2gamma in all seminomatous GCT. In contrast, the staining pattern with antibodies to c-KIT, PLAP and OCT3/4 was variable or absent. The lowest specificity was obtained with c-KIT, which was expressed in a variety of non-GCT. The only M2A+ mesothelioma expressed no other seminoma markers. No correlation between serum PLAP level and tissue PLAP expression was found., Conclusions: M2A and AP-2gamma are the most sensitive markers for seminoma metastases or primary extragonadal seminomas. Combination of these markers provides highly specific and clear results for detection of a seminomatous GCT.

Objective: Pure pineal germinomas have been rarely reported in girls. Gender incidence and differences of pure pineal germinomas are not well known. The authors report a series of pure pineal germinoma and its gender characteristic is reviewed., Methods and Results: Of a total of 50 germ cell tumors operated on between 1988 and 2004 we found 26 cases (median age at diagnosis, 12 years) of pineal germ cell tumors. Of these, 14 cases (male/female ratio: 13/1) were pure pineal germinomas, and 12 cases (male/female ratio: 12/0) were non-germinoma germ cell tumors. In pure pineal germinomas, the main clinical presentations were intracranial hypertension and cranial nerve dysfunction. Imaging studies disclosed a homogeneous type of tumor (n = 10) and associated hydrocephalus (n = 6). Cases were managed with biopsy and subsequent radiation therapy and chemotherapy. After a follow up of 10 years, pure germinoma cases have no neurological deficits and tumor recurrence. The literature on gender incidence of pure pineal germinomas is analyzed and possible causes are discussed., Conclusions: Although rare, pure pineal germinoma can be found in female subjects. On the basis of the literature review, the male/female ratio in cases of pure pineal germinoma is between 5:1 and 22:1 (mean 14:1). In our series, the male/female ratio was 13:1.

Objective: Evaluation of results of the neurofiberscopic biopsy of tumors of the pineal region and posterior third ventricle., Methods: From 2001 to 2004, 23 patients (mean age, 30.6 yr) with tumors located in the pineal region or posterior third ventricle underwent neurofiberscopic biopsy with simultaneous third ventriculostomy. The procedure was indicated for verification of the histological diagnosis of the neoplasm, which was planned to be treated by radiotherapy and/or chemotherapy without open surgery (eight patients), establishment of the pathological diagnosis for further choice of the most appropriate treatment strategy (11 patients), differentiation of the recurrent neoplasm and radiation necrosis (two patients), and decompression of the large tumor-associated cyst (two patients). In six previously shunted patients, substitution of the ventriculoperitoneal shunt on the third ventricle stoma was performed., Results: There was no postoperative mortality or permanent morbidity. In all cases, the obtained tissue sample was sufficient for pathological diagnosis. Transient postoperative complications included fever (15 patients), nausea and vomiting (three patients), and diplopia (one patient). On the long-term follow-up, delayed third ventricular stoma failure caused by tumor regrowth and scar formation was found in one patient, and dissemination of the malignant glioma through the subarachnoid space was found in another patient., Conclusion: Neurofiberscopic biopsy represents a useful method for sampling of tumors of the pineal region and posterior third ventricle, which can be effectively used in both previously shunted and shunt-free patients.

Several types of tumors are known to originate from the pineal region, among them pineal parenchymal tumors (PPTs) and papillary tumors of the pineal region (PTPRs), probably derived from the subcommissural organ. As a result of their rarity, their histologic diagnosis remains difficult. To identify molecular markers, using CodeLink oligonucleotide arrays, gene expression was studied in 3 PPTs (2 pineocytomas and one pineoblastoma), 2 PTPRs, and one chordoid glioma, another rare tumor of the third ventricle. Because PTPR and chordoid glioma may present ependymal differentiation, gene expression was also analyzed in 4 ependymomas. The gene patterns of the 3 PPTs fell in the same cluster. The pineocytomas showed high expression of TPH, HIOMT, and genes related to phototransduction in the retina (OPN4, RGS16, and CRB3), whereas the pineoblastoma showed high expression of UBEC2, SOX4, TERT, TEP1, PRAME, CD24, POU4F2, and HOXD13. Using reverse transcriptase-polymerase chain reaction on 13 PPTs, we demonstrated that PRAME, CD24, POU4F2, and HOXD13 might be candidates for grading PPT with intermediate differentiation. PTPRs, classified with chordoid glioma and separately from ependymomas, showed high expression of SPEDF, KRT18, and genes encoding proteins reported to be expressed in the subcommissural organ, namely ZFH4, RFX3, TTR, and CGRP. Our results highlight the usefulness of gene expression profiling for classify tumors of the pineal region and identify genes with potential use as diagnostic markers.

This two part series of articles presents poignant narratives based on interviews done with two mothers who suffered the worst pain imaginable--the pain of losing a child. Each woman describes an insensitive health care system with providers unaware of parental views and needs when confronted with difficult decisions during their children's illnesses and subsequent deaths. Each woman also hoped that telling her story might help nurses and other health care providers better understand how to help. Part 1 of the series is the story of a professional, highly educated mother who experienced periods of extreme distress while she tried to give her premature daughter an identity and meaningful life in the neonatal intensive care unit and the difficulties faced when she was transferred to another unit as she became more ill Part 2 of the series is the story of a highly devoted working mother who tried to understand the information that was rarely offered to her as she lived through the extreme surgical and uncertain medical interventions given her toddler daughter with a fast-growing brain tumor. Both women faced hard decisions along their daughters' illness trajectories. Both hoped for support from their health care providers in being active in decision-making. These narratives are in their own voices, from their own points of view.

Microsurgical resection remains the mainstay of treatment for truly benign brain tumors that can be safely resected because of the potential for permanent cure with most histologic findings, including most of the histologic findings discussed in this article. Physicians must keep in mind the indolent nature of many of the benign brain tumors and realize that many patients are likely to live out normal life spans if tumor control is achieved. Therefore, it is not sufficient simply to consider local tumor control rates and short-term toxicity risks when choosing between surgery, stereotactic radiosurgery, and fractionated radiotherapy. Patients need to be apprised of all therapeutic options and to make their decisions with all information required to evaluate the risks and benefits. For benign brain tumors, these decisions may have consequences that last for decades.